Cloudy with a Chance of Autoimmunity

Sometimes everything gets screwed up and it's all for the best.

My 2yo son Dibbs was screened for celiac disease and for celiac genetic markers at the same time his big sister was in July 2013 through a free screening session by a team from the Center for Celiac Research out of Mass General Hospital for Children.

In late August, we learned in a letter that Dibbs was negative for antibodies and that Sweetie was positive. By the end of September, we had her official biopsy-confirmed diagnosis. By the end of December, we had her (and my) genetic test results, but not his. I wrote to and called the research clinic and explained my urgency to know whether or not my baby also had the gene we have. They told me that, as soon as they had more testing kits arrive, they would expedite his test results and email me since his sister was a confirmed diagnosis.

But in February, I received an email saying that the original letter stating that his celiac antibody test was negative should never have been sent. They did not have any record of actually performing the test and did not appear to have a sample on which they could perform the genetic testing either. His blood sample was just not there.

Now, this is where I should say that, while I was angry to have learned I received false results, the Center for Celiac Research has been very communicative and helpful in exploring what happened. They recommended I have my son re-screened elsewhere and sent the case to a review board.

My son's pediatrician ordered a celiac panel for us last week. A celiac panel consists of at least these 4 tests:

1. tTG (anti-tissue transglutaminase)-IgA
2. EMA (anti-endomysial antibodies)-IgA
3. IgG
4. Total serum IgA

Now, the reason you need all of them is that, if #1 alone is positive, it could be a false positive if you also have another autoimmune disease (like, say, type 1 diabetes). If #2 is negative, it could just be that you don't have enough intestinal damage for it to show as positive yet. Test #3 is kind of a backup test for a different form of antibody, but it's not nearly as specific for celiac. And if you're found in test #4 to have a deficiency in the amount of IgA antibody you're able to produce, well then, you may as well just discount the whole lot of it.

For the record, Sweetie's panel results as run by Children's Medical Center (with standard reference range they gave us in parentheses):

1. tTG IgA: >128 (normal range <10)
2. EMA IgA: 1:80 (normal range 1:10)
3. IgG: 1 (normal range <10)
4. total serum IgA: 152 (normal range 46-345)

Guess where Dibbs' problem was though. That's right. A problem with Test #4. He is apparently IgA deficient. His total serum IgA clocked in at...10.

This is from the page on celiac testing at About.com:

If you have a very low total serum IgA, that can invalidate the three blood tests that rely on your IgA levels. People with celiac disease suffer from low total IgA levels about 10 to 15 times more frequently than people in the general population.

Now, Dibbs has no symptoms for celiac, we don't have a genetic result for him to know if he's susceptible, and the other three tests came back negative/normal range. So that's good, right? Except for the whole "invalidate" part. Our pediatrician has deemed the panel "inconclusive" for this reason.

IgA deficiency also, however, according to my friend Wikipedia, has "an association with autoimmune disease" with patients having "a higher risk of developing autoimmune diseases in middle age."

Well, crap. Seems the crosshairs are on my little fella as well.

But here's the silver lining as I see it.

First of all, nothing seems wrong with the boy, and of course we're glad of that. :)

Secondly, I've since discovered, in all my communication with the research coordinator who did or did not run the original test, that their clinic tests only for tTG IgA. They would never have run the total serum IgA test for this research study to know that he was deficient.

So had they actually run his sample, I'd still have received a negative result. But I wouldn't know that I couldn't trust it.

If nothing had gone wrong with Dibbs's screening, I'd have had no reason to have him re-screened. I would not have suspected IgA deficiency. I would not have understood that the Center for Celiac Research runs only the 1st component of the panel. I would not have any of the information I currently have.

But I'd have the HLA genetic marker results...which I still intend to get.

In the meantime, like I said, he's asymptomatic. I hate to think that I'm continuing to expose someone to gluten who may be likely to present with celiac or even that I might somehow increase the odds of his developing an autoimmune disease by the choices we make now, but here's the thing...

As Hubster and I always say, you do the best you can with the information you have in the moment. We are already lightyears ahead of where were with our daughter who was a year and a half older before she was diagnosed. We are already expertly prepared to deal with diabetes and celiac. And if all Dibbs is is IgA deficient (one hopes), it's mostly harmless and there's nothing to be done about it.

As Wendy relayed from Dr. Fasano in a post that gave me comfort after my daughter's diagnosis, "you can't go back; you can only move forward."

So forward we go.

Dibbs: Two Years

My baby. We decided you were terribly two about two months ago, but as of yesterday, you are official.

You are absolutely adibbable.

You are lovey, huggable, incredibly thoughtful. You are everyone's friend and Mommy's little helper, standing at the sink washing dishes (which is really just filling cups of water and dumping them).

Single Handedly

I have to give a shout-out to Asante for a benefit I hadn't considered when I started their Snap pump in December.

On Monday, I knew I needed to change out my pump body before lunch (3 units remaining), but between the cranky toddler and the gluten-free food prep for the preschooler, I forgot. I made lunch, we ate quickly, and Dibbs climbed into my lap and passed out on my shoulder. Then my daily alert went off (thanks, Snap). I'd forgotten to bolus for lunch, too, apparently. Yikes!

Now I've got a sleeping two year old nuzzling my neck and I need to bolus insulin and I don't have enough left in the reservoir to cover my lunch. I needed to change out the whole thing.

Wish I could say that this kind of thing doesn't happen a lot, but, you know, kids.

No worries. I've got this. With one hand tied behind my...toddler.

Between the perforated packaging on the pump body, the self-priming tubing, and the one-button release for the cartridge/controller connection, I did it all while never disturbing the little boy in my arms. There was no reservoir to fill, no syringe to handle. Just a glass insulin cartridge to pop in. I did it all with only one hand.

Well done, Asante. From an exhausted mom...and a well-rested two year old.

The Thicker Envelope

I don't normally take both of the kids to the supermarket with me. If you knew Lord Fussington and Lady Runsalot, you'd understand. But on Friday afternoon, I loaded them into the car for my weekly trip.

And on the way out, I stopped to check the mail.

Three envelopes. Addressed to myself, Sweetie, and Dibbs.

One envelope was thicker.

Like many of us who attended the Friends for Life conference, I took advantage of screening opportunities. I had a retinal screening for myself. I had the kids screened for type 1 diabetes markers:

Both kids tested negative this year. I found out a couple weeks ago. Whew.

And I had myself and both kids screened for the antibodies for Celiac disease, as well as for the genetic HLA markers for Celiac.

The screening day was an awful day - our worst at the conference. My daughter was cranky, tired, constipated, and in an overwhelming environment with lots of people. She had an epic tantrum with my husband in the lobby (in front of many people) while I attempted to fill out all of our screening paperwork with Dr. Fasano. I had to sit at a laptop and fill out the same multi-page questionnaire with hundreds of questions...THREE times. Once for each of us. My husband returned her to me and they were both spent. Worked up. Done for the day. That's the state we were all in as we dragged the kids in for the blood draw.

I went first. Easy peasy. But it took 3 or 4 technicians to hold Sweetie as they did the draw. She's super strong. She cried. She tried to get away. Having them do my son was even worse. He'd never ever been restrained before. I held his head as they pierced the crook of his pristine chubby arm. I held him still with every ounce of my strength, wrapped both arms and legs around his head and body...and I flipped out inside when they stuck the second arm to try to get enough blood.

When it was over, each kid got a stuffed frog and settled into the double stroller as we made our way back to our hotel room. They were immediately fine. Fully recovered.

I was shell-shocked.

I didn't say a word. Didn't want to talk to Hubster. My arms were stuck straight in front of me pushing the stroller, unbending, still feeling the muscles flexed as though I still held my son against me so he couldn't move. I got back to the room and just lost it.

I don't ever want to hold my own child down like that again.

But the kids? They were fine.

Dr. Fasano passed us in the hallway later that week and I stopped him. I apologized for my kids' behavior and for my nerves that day. Thanked him for the research he does on Celiac disease.

Nearly two months later, that thicker envelope happens to be for Sweetie.

Dibbs: One Year

Son,

Oh wow. Every time I say I have a son, something goes all over me. I have a SON.

It's tough to say no to this face.

Well, boy, you have reached it. The end of infancy. The one year mark. You're almost a toddler.

One

I say 'almost' because you're still only cruising along my furniture rather than walking. But you can hustle up a staircase faster than the pets you're chasing, so it's only a matter of time. We can usually hear you crawling through the house, a plastic toy in each hand. Click, clack, click, clack. The faster the clicks, the more mischief you're up to.

A New Take on Twinkle

Hubster heard the little ones and me laughing so hard that I couldn't catch my breath, so he came in to see what all the fuss was about and I asked him to take video...

It seems I've found a fun, new way to sing Sweetie's favorite song. A little more her style, too.

boop boop boop boop!

Hotel for Tots

In the course of our travels, we've learned a lot about how to keep babies and toddlers safe and happy in a hotel.

Mitigating Disaster

Unplug the phone from the wall. It is a fascinating toy with voices at the press of a button. "Hello? How are you, Dada?" We have cell phones. And if I need to call guest services, I just replug and dial.

Glass glasses are replaced daily by Housekeeping and should go up and away. At one point, I thought they made great bathtime rinse cups. Then my two year old smashed her hand into one and shattered it. Bring bandages. And just use a sippy cup for rinsing.

Diapers. Dealing with diapers in a hotel can be tricky. Hotels don't have diaper pails. I've yet to find the perfect solution for stinky diapers. There's the individual bag method (I can still smell it). There's the "see if the housekeeping cart is parked down the hall" approach. You could bring an odor proof wetbag (made for cloth diapers) or some kind of sealable box. If you figure out a great method, I'd love to hear it. On cruises, most lines will actually provide a pail. I like cruises.

Most hotels offer roll away beds and cribs. Our kiddos share the bed. Bring a waterproof crib sheet to throw underneath them. In a dozen or more hotel trips with babies, we've soiled one set of hotel sheets. Not bad, says I.

Stationery items like pens, notepads, hotel booklets, and the Gideon good book are best kept in a hidden place, too. It is fun to draw on walls and shred paper when you are two.

Sweetie was approximately 20 months when she was tall enough to reach the big red button on the elevator panel at a Holiday Inn in Amarillo. The emergency responder dispatcher was very friendly.

Windows are awesome to stand in. Make sure that they don't open and that your child's life insurance policy is up to date. Our suite at the Hotel Marlowe in Boston had a gorgeous window that you could stand in that overlooked a busy street by the Charles river and a big golden telescope that you could knock over right in the window. I aged on that trip. Our room at the Parc 55 Wyndham in San Francisco had a little air vent you could open (pictured under the pig). We nearly lost all four Little Einsteins from our room on the 18th floor.

All the Comforts of Home

When you get to the room, lay out all of the toys and snap a quick photo. When you're packing later, you'll appreciate the visual as you go looking under the bed and dresser for that seventh stacking cup. In two and a half years, we've lost only two pacifiers and one wooden peg. Always check for toys who have gone off on little adventures of their own, too. I couldn't find Elly and Pocoyo all morning one day...they were people watching in this window at the Holiday Inn.

You never know what extra little amenities a hotel may be able to provide. A nice young man at the front desk of a Holiday Inn in the Boston area unlocked the fridge in the breakfast area every time I wanted a carton of milk for bedtime. At the Omni in Philadelphia, we were given two little backpacks with magnifying glasses, hackey sack balls, and coloring books. At Orlando's Marriott World Center, we were offered use of a stroller during our stay from their cache of abandoned strollers. The ladies in the gift shop at San Francisco's Holiday Inn Golden Gateway offered Sweetie two Dum-Dums every time we came in. And at the Parc 55 Wyndham in San Francisco, we got four stuffed barnyard animals from the front desk. And band-aids.

Snacks and drinks are challenging when you travel. It's easy to find yourself dehydrated. Most hotel rooms have fridges or can provide one at no extra charge. We like to make a drugstore trip to stock up on drinks, diapers, and snack foods once we get in. (Thanks a million to Sarah for bringing us juice, water, and goldfish!) Bonus: shopping bags make great dirty clothes sacks.

One item I am hardly ever traveling without is a small bottle (10oz or less) of dish soap in a ziploc bag. I wash sippy cups, pacifiers, toys, and occasionally even clothes in the bathroom sink.

The world is not baby-proofed. And though I keep offering my Sweetie out as a test subject to people beginning their baby-proofing process, it will still be my job to keep her and her brother happy and safe when we are out in the world. I can assure you - it's a full-time job.

Wordless Wednesday: Slap Happy

Three for Thursday: Dibbs in the Wild

First time sitting in the grass.

My Fault

"It's not your fault," says the physical therapist as she shows us the photos they've taken of the baby's head.

I nod. It's my fault. My face betrays nothing of what I'm thinking.

"It's nothing you did," she smiles reassuringly.

It's something I did, I think. Or didn't do. It must be.

I think of the repositioning tactics I tried but how he still insisted on sleeping on his right side. I think of how many times I failed to notice him preferring turning to his right. How I didn't have torticollis on my radar because he seemed to have full range of motion in his neck.

Dibbs getting his DSI (imaging) for his helmet.
He licked the stocking on his face. And drooled a lot.

"No, it's just that he was a big baby and he didn't have a lot of space in utero. It happens."

Aaaaaaand...there it is. The kicker. This woman doesn't know that I have type 1 diabetes. Doesn't know that "big baby" is one of the many phrases we tire of blaming ourselves for. And it's all the ammunition I need to metaphorically shoot myself with the guilt gun.

So how did we do?

That's a great question.

Pre-Operation:

My Blood Sugar: flatlined for 12 hours between 108-117 leading up to c-section (see 6 hr graph above). Perfection!

My Pump: removed just before surgery and replaced with an IV insulin drip

My Insulin: They put me on a Novolin R drip of 1 unit/hour. (Really? R?) They also started a D5 (dextrose) drip. Yin and Yang!

My Blood Pressure: All over the place - the systolic would be too high, then just the diastolic. Then it would normalize. Nervous much? My blood pressure never had a problem during either pregnancy, but before both surgeries, this happened.

Epidural went in easily, but before they pushed any Fentanyl through it, I was struggling with horrible nausea. My nurse and anesthesiologist determined it was from being flat on my back under the unusually large weight of my uterus. Once they dosed the Fentanyl, I got the shakes something fierce. (This is one of many reasons why I don't regularly do opioids.)

During the Operation:

My Blood Sugar: I lay with my arms out to each side, BP cuff on right arm and Dexcom receiver in my left hand where I could check it as I liked. My BG immediately started climbing. Before the incision was made, I was 134 and climbing. As they sewed me up, 169.

My Pump: I was missing it.

My Insulin: R is a joke. I should have put some of my own insulin on board before disconnect. If Apidra/Humalog/Novolog are jet airplanes, R is a kid on a bicycle.

My Blood Pressure: It was normal throughout.

The nausea from the weight of my belly as I lay on my back was so overwhelming that I requested a vomit bag and had to use it. They pushed some Zofran through my IV and that helped, but honestly, I just needed the kid and all his fluid OUT. This time the epidural knocked out any sensation from my ribs down. I felt even less than I remembered feeling during Sweetie's birth. The catheter didn't tug or hurt like before. It was just...easy, quick, painless. There was immediate relief when they removed my 10 1/2 pound son and they couldn't stop talking about the large volume of fluid in there with him.

Post-Op:

He was born at 8:23 and, by 8:51, he was skin to skin with me being wheeled into post-op where we were encouraged (!) to immediately start breastfeeding.

He seemed a little jittery, so I was curious about his BG, but when they finally tested it, it was 50. Totally newborn normal. (Sweetie had been 54 at birth.)

We were held up in post-op for a couple of hours while they got a room ready.

Because of my recent MRSA infection, there were lots of "contact isolation" procedures they had to follow as far as our room was concerned. Throughout the week, every hospital employee who entered had to wear a plastic gown and rubber gloves. Infectious Diseases says I can be retested a year after my infection, and after three negative tests, I'll be scarlet letter free, so to speak. Until then, plaguesville, population me.

My Blood Sugar: By 9am, I was 196. 10am, 236. 11am, 224.

My Insulin: I took an injection of 5u Apidra (secretly) to correct the 235. But as I continued to hold there over the next hour, it was clear to me that I needed to trash the dumb bag of R and get my own Apidra going.

My Pump: At 11am, I filled a pod and kick-started my Omnipod PDM at my new basal rate. I also bolused another 2u of correction. By the time we got to our room around noon, I was 183. Better.

I could give you all a lot more details about my management through my stay (see log below), but the long and the short of it is that, once I brought the post-op high down, my blood sugars stayed between 68 and 173 the whole hospital stay. My goal was between 80 and 200, so I feel great about that.

The nurses ranged from curious to impressed to downright marveled in response to my continuous glucose monitor (Dexcom Seven+). My L&D nurse wanted it right by me throughout surgery rather than have my husband hang on to it, and my postpartum nurses would bring other nurses in to see it.

They were a little unsure what to make of my self-monitoring and pumping though. They all wanted to stay abreast of my most recent BG reading, but some nurses bugged me about how much I was bolusing and when. I felt less comfortable sharing that - probably because I regularly 'adjust' suggested dosages based on my CGM graph trends, how I feel at the moment, or whether or not I trust the aggressiveness of a particular correction factor. And I was also tweaking basal insulin rates as necessary each day on the advice of my endo (who stopped by my room daily).

The only nurse who drove me CRAZY was Nurse Chattypants. She was so insistent that I touch my incision scar that she grabbed my fingers and forcibly jammed them along my pubic line - um, thanks, but NOT cool. Anyway, she comes in for her first shift with me and rolls me on my side saying she needs to inspect my pump dressing (pod was on my lower back). I was like "what the HELL??!!" and then she kept asking if I needed her to tape it up for my shower. She's so lucky that I don't punch people in the face as a general rule. I told her that my diabetes care was my concern and that I trusted my nurses to be there to care for my postpartum needs. Didn't stop her though. I was in her care twice during my stay for 12 hrs each time. She. Never. Shut. Up.

When I called to place my first dinner order to the Dining Services desk and asked about meal delivery times so that I could time my insulin, the dining crew asked if my doctor meant to order me the "diabetic diet." I skipped the teaching opportunity, rolled my eyes and laughed. "No, I'm breastfeeding. Thanks. The regular menu order is correct." It was skimpy to begin with - can't imagine what their idea of "diabetic diet" was.

Everyone but me and my endocrinologist seemed concerned about whether my diabetes was "stable" after having the baby. Even despite blood sugar values that were almost non-diabetic. When I asked when the earliest I could be discharged was, even my OB said it depended on what Dr. M thought. When I told Dr. M that, she laughed. My numbers were boringly stable (um...no highs AND no lows - practically cured) and, as usual, there was nothing to indicate I couldn't roll with it if they weren't. I had less swelling in my feet than anyone else on my recovery floor. I was lucid, responsive, wearing regular clothes. I was walking the halls and to the nurses' desk, even when my epidural had fallen out and I didn't realize it.

Come ON, freaking hospital people. Stop micromanaging shit you have no more than a cursory textbook understanding of and watch a HOSS living it.

We eat like you, we usually test our blood sugars WAY more often than you think we should, and I resent your help. It's much more complicated than "Oh, you're on insulin? What's your dose?" Asked of me twice. Ummm...how much time have you got? I'll explain my four basal rates, my four variable insulin:carb ratios, my three target glucose levels by time of day, and the four different correction sensitivity factors. Then we'll take my average total daily dosage and discuss the average bolus/basal breakdown by percentage, factoring in the difference between correction bolus and meal bolus. After that, we will talk about how my faster acting analog that none of you have ever heard of peaks differently than the insulin you're used to dosing here and at no point will I allow you to even touch my pump. Got it? Now ask me again how much insulin I take as though it's a pill I swallow before meals.

Anyway, the staff at Dallas Presby really did take excellent care of me. The surgery was quick and painless, the recovery is going well, and I'm home with my 1-week-old and Hubster and Sweetie where I belong.

My Current Blood Sugar: 73 and steady for the last hour with an anticipated low in another 1-2hrs due to recent breastfeeding session. Snack is imminent.

My Pump: pluggin' away

My Insulin: currently running a basal of .90u per hour, which I lowered 10% from where it was two days ago due to going through half a bottle of glucose tablets in a day

My Blood Pressure: eh, feels okay to me